Cholangiocarcinomas (CCAs) are cancers that arise in the peripheral or central bile ducts. They have substantial heterogeneity in their etiologies and histopathologic phenotypes, ranging from cholangiolar carcinomas arising from the smallest bile ducts or from dedifferentiating hepatocytes through cellular plasticity, to ductular cancers in medium and large-sized bile ducts. The surgical delineation of intrahepatic, perihilar, and distal CCAs does not match the biological classification, as intrahepatic CCAs include both small duct cholangiolar carcinoma and intermediate duct ductular or mucinous CCAs, which have different genomic and genetic characteristics.
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